Pregnancy in women with Marfan’s Syndrome

Medical Management of Marfan Syndrome

“…It is not surprising, therefore, that a majority of the aortic complications in pregnant Marfan syndrome patients have been described in the third trimester of pregnancy. 69 Interestingly, the risk of aortic rupture and dissection extends beyond the prepartum and peripartum time periods, with up to one fifth of complications occurring Ն2 days after uneventful delivery. 69 Risk of aortic complications during pregnancy is directly related to the degree of baseline aortic enlargement of the aortic root, with few complications occurring in those with aortic root diameters Ͻ4.2 cm.…”

“…69 Interestingly, the risk of aortic rupture and dissection extends beyond the prepartum and peripartum time periods, with up to one fifth of complications occurring Ն2 days after uneventful delivery. 69 Risk of aortic complications during pregnancy is directly related to the degree of baseline aortic enlargement of the aortic root, with few complications occurring in those with aortic root diameters Ͻ4.2 cm. 70,71 The most common aortic complication in those with minimal root dilatation is type B dissection.…”

“…3 Prophylactic treatment with ␤-blockers can blunt increases in heart rate and dP/dt from mid-trimester on, albeit with small risks of fetal intrauterine growth restriction, hyperbilirubinemia, and hyperglycemia. 69 Angiotensin receptor blockade is contraindicated in women attempting to conceive and during pregnancy. Periodic echocardiographic assessment of aortic root size is recommended to identify those at high risk for complications.…”

“…Labor and delivery by the vaginal route are likely safe, with appropriate regional anesthesia and positioning to reduce fluctuations of venous return and pulse pressure during delivery. 69 In those patients with aortic root diameters much Ͼ4.0 cm, consideration can be given to prophylactic root repair with sparing of the native aortic valve, as described subsequently. Composite graft repair with a mechanical valve is problematic with pregnancy because of the need for long-term warfarin therapy, which can be teratogenic.…”

Cardiovascular Problems in Pregnant Women With Marfan Syndrome

“…Pregnancy is associated with increased risk of aortic dissection, 3,4 probably caused by hemodynamic changes 5 and by hormonally mediated decrease in the amount of mucopolysaccharides and loss of elastic fibers in the aortic wall. 6 Reviewing the English literature in the last decade, we found 39 cases that provide information regarding potential pregnancy-related complications in women with MFS.…”